Lichen Sclerosus

Epidemiology & Risk Factors

• Bimodal age distribution: Peak in postmenopausal women (most common); secondary peak in prepubertal girls.
• Autoimmune etiology: Associated w/ thyroiditis, vitiligo, pernicious anemia, type 1 DM.

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Clinical Features

• History: Severe vulvar pruritus (worse at night), dyspareunia, dysuria, painful defecation/anal fissures.
• Physical Exam:
  • Porcelain-white papules/plaques w/ skin atrophy (“cigarette-paper” appearance). c
  • Anogenital distribution in “figure-of-eight” pattern surrounding vulva and anus.
  • Architectural distortion: Fused labia minora, clitoral phimosis, introital stenosis.
  • Hemorrhagic purpura, ecchymoses, fissures.
  • Sparing of vaginal mucosa (crucial distinguishing feature).

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Diagnosis

• Initial/Clinical: Visual inspection based on characteristic porcelain-white atrophy and figure-of-eight distribution.
• Confirmatory/Gold Standard: Vulvar punch biopsy.
  • Indicated if: Diagnosis uncertain, atypical presentation, unresponsive to topical steroids, or suspicious for malignancy.
  • Histology: Epidermal atrophy, hyperkeratosis, dermal hyalinization, band-like lymphocytic infiltrate.

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Differential Diagnostics

• Lichen Planus: Involves vaginal mucosa (desquamative vaginitis, friable mucosa), oral mucosa (Wickham striae), purple polygonal pruritic papules.
• Vulvovaginal Atrophy: Pale/dry mucosa, loss of rugae, involves vagina; lacks figure-of-eight lesions, structural resorption, or porcelain-white plaques; responds to topical estrogen.
• Lichen Simplex Chronicus: Thickened leathery hyperpigmented plaques (lichenification) 2/2 repetitive scratching; no skin atrophy or hypopigmentation.
• Vulvovaginal Candidiasis: Thick “cottage-cheese” discharge, pseudohyphae on KOH prep; no structural loss or chronic depigmentation.

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Management

1. First-line: High-potency topical corticosteroids (e.g., Clobetasol propionate 0.05% ointment daily x 6–12 weeks, then taper to maintenance dose).
2. Second-line: Topical calcineurin inhibitors (e.g., Tacrolimus, Pimecrolimus) for steroid-refractory or intolerant pts.
3. Refractory/Surgical: Surgical reconstruction only for introital stenosis causing functional impairment (contraindicated for active inflammation).

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Complications

• Squamous Cell Carcinoma (SCC) of the vulva (4–5% risk; requires long-term clinical surveillance and repeat biopsy for non-healing/ulcerated lesions). c
• Introital stenosis and permanent loss of vulvar anatomy.
• Sexual dysfunction and severe dyspareunia.